Thyroglossal Duct Cyst

Introduction
Thyroglossal duct cysts are the most frequently occurring congenital cervical anomalies,
with a 7% population prevalence. They can form anywhere along the thyroid’s route of
migration from the tongue base to the inferior neck. They often present as midline neck
cysts closely associated with the hyoid bone.[1]
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Etiology
A thyroglossal duct cyst is an embryologic remnant that forms due to the failure of
closure of the thyroglossal duct extending from the foramen cecum in the tongue to the
thyroid’s location in the neck. The thyroid begins to develop in the third week of
gestation as a median outgrowth from the primitive pharynx. The thyroid primordium
originates at the foramen cecum at the junction of the anterior two-thirds and posterior
one-third of the tongue. From there, the thyroid descends to the neck, passing anterior
and in close relation to the developing hyoid bone. It reaches its final position in the
inferior pre-tracheal neck by the seventh week of gestation.
The thyroglossal duct is the narrow tubular structure left from the thyroid’s descent and
connects the thyroid gland to the foramen cecum. The distal part of the duct differentiates
into the pyramidal lobe of the thyroid gland in about 50% of people. The thyroglossal
duct normally involutes by the tenth week of gestation. If any portion of the duct persists,
secretion from the epithelial lining can result in inflammation and thyroglossal duct cyst
formation.[2][3]
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Epidemiology
Thyroglossal duct cysts are present in about 7% of the population worldwide. They have
an equal preponderance between male and female individuals. Although they are known
to be the most common pediatric mass, they also present in adults with varying
frequency. These types of cysts are closely associated with the hyoid bone. They are
about 20% to 25% present at the level of suprahyoid, 15% to 20% present at the level of
hyoid, and 25% to 65 % present at the infrahyoid level.[4][5]
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Histopathology
Thyroglossal duct cysts are cystic structures lined by respiratory epithelium, squamous
epithelium, or a combination of both. Due to a high frequency of infection, inflammatory
infiltrates can be present. These can appear as granulation tissue or giant cells. In about
70% of cases, microscopic foci of ectopic thyroid gland tissue can be found, usually
within the cyst wall.
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History and Physical

Thyroglossal duct cysts typically present as mobile midline neck masses near the hyoid
bone. They often are asymptomatic. However, they can present as an abscess or
intermittently draining sinus. The mass will elevate with tongue protrusion or
swallowing. The mass is closely associated with the hyoid bone and most commonly
found at or below the level of the hyoid.
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Evaluation
Imaging should be performed to both diagnose the thyroglossal duct cyst and evaluate for
the presence of healthy thyroid tissue. If normal thyroid tissue in the inferior neck is
absent, the patient and/or parents should receive counseling on the possibility of lifelong
thyroid replacement therapy after surgery.
Ultrasound is the ideal initial imaging choice. Ultrasound is readily available,
inexpensive, and noninvasive. It does not require ionizing radiation or sedation, which is
important in treating children. CT scans and MRI can be used to evaluate thyroglossal
duct cysts and the presence of normal thyroid tissue, but ultrasound alone in usually
sufficient.
Some surgeons advocate for routine thyroid function testing preoperatively. This may be
helpful if ectopic thyroid tissue is expected, but the literature does not support routine lab
work for uncomplicated thyroglossal duct cysts.[6][3][7]
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Treatment / Management
The treatment for thyroglossal duct cysts is surgical removal to prevent recurrent
infections due to the small risk of malignancy. Simple excision of thyroglossal duct
cysts is associated with high recurrence rates (45% to 55%). The Sistrunk operation is
considered the standard of surgical management and has dramatically reduced recurrence
rates. This procedure requires a more extensive surgical resection including the central
third of the hyoid bone and a core of base of tongue tissue.[8][9]
The Sistrunk procedure should not be performed in the setting of acute infection. The
patient should receive systemic antibiotics and removal should be planned after the
infection has resolved. If preoperative evaluation reveals no other functional thyroid
tissue, removal can still be performed with the acknowledgment that hormone
replacement therapy may be necessary postoperatively.
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Differential Diagnosis
The differential diagnosis of thyroglossal duct cysts includes midline neck masses and
cystic neck masses, as well as cystic metastatic lymph nodes, dermoid or epidermoid
cysts, and second branchial cleft cysts. Cystic metastatic lymph nodes usually originate
from either papillary thyroid carcinomas or squamous cell carcinomas of the upper
aerodigestive tract. Dermoid or epidermoid cysts can also be midline cystic neck masses.
The close relationship of thyroglossal duct cysts with the hyoid bone is a key feature for
differentiation. However, the final differentiation is often not made until pathologic
diagnosis. Second branchial cleft cysts are also anterior neck cystic masses. However,
branchial cleft cysts are lateral and not associated with the hyoid bone.
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Surgical Oncology
Less than 1% of thyroglossal duct cysts develop into a carcinoma. Papillary carcinoma is
the most common malignancy found (92.1%) followed by squamous cell carcinoma
(4.3%). Thyroglossal duct cyst carcinoma typically presents with an asymptomatic
midline neck mass. 73.3% of these types of carcinomas were diagnosed as an incidental
finding on final pathologic analysis. Patients diagnosed with thyroglossal duct cyst
carcinoma tend to be adults and have an older average age than the typical thyroglossal
duct cyst patient. Treatment of thyroglossal duct cyst papillary carcinoma involves a
Sistrunk procedure followed by evaluation of lateral neck lymph nodes and thyroid. Total
thyroidectomy, lateral neck dissection and/or radioactive iodine may be indicated
depending on the extent of disease. Overall prognosis is excellent, with a survival rate of
99.4% and a recurrence rate of 4.3%.
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Prognosis
Following the Sistrunk procedure, the prognosis is usually excellent. About 10% of
thyroglossal duct cysts recur after Sistrunk. There is a much higher recurrence rate with
simple excision without excising the middle third of the hyoid bone. 1% of thyroglossal
duct cysts are malignant, which is usually diagnosed after surgical removal.
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Complications
The most common complication of the Sistrunk procedure is a recurrence of the
thyroglossal duct cyst, which occurs in about 10% of cases. Contributing factors to
recurrence include incomplete excision, intraoperative rupture, surgical proficiency and
experience, and presence of infection. However, recurrence can still occur after
technically proficient procedures.
A laryngotracheal injury is a rare and potentially devastating complication of the Sistrunk
procedure, resulting in issues with the airway, swallowing, and/or voice. It can be caused
by erroneous resection of the thyroid cartilage instead of the hyoid bone. Appropriate
identification of the hyoid bone, thyroid cartilage, and the thyrohyoid membrane is
essential to prevent this during surgery.
A hypoglossal injury is also rare but has been reported after the Sistrunk procedure,
resulting in paralysis of half of the tongue. The hypoglossal nerve travels lateral to the
hyoglossus muscle and medial to the stylohyoid muscle and lingual nerve near the lateral
portion of the hyoid bone. It is important to keep the hyoid resection medial to the lesser
cornu of the hyoid to avoid hypoglossal injury.
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Postoperative and Rehabilitation Care

Following the Sistrunk procedure, patients are instructed to avoid heavy lifting for 2 to 6
weeks. Depending on the size of the thyroglossal duct cyst, there may be a surgical drain
in place, which would be removed within a few days of surgery. Pain medication or
antibiotics may be prescribed postoperatively. Patients can usually return to work or
school 1 week after surgery.
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Enhancing Healthcare Team Outcomes

Thyroglossal duct cyst is managed by an interprofessional team that consists of a
pediatrician, primary care provider, nurse practitioner, and a surgeon. The exact
incidence of thyroglossal duct cyst remains debatable, but one fact is certain; it is more
common in children compared to adults. It usually presents as a midline neck swelling
and the standard treatment is the Sistrunk procedure. This procedure is associated with
good outcomes and low recurrence rate of 3-5%. Very few complications have been
reported in the literature, and most children have no residual sequelae.[10][11]